The Effect of Hypoxic Gas Therapy Using Nitrogen Before Bilateral Pulmonary Artery Banding on Hemodynamics and Pulmonary Artery Development.

This study aimed to identify its influence on pulmonary development and hemodynamics before bidirectional Glenn (BDG) anastomosis. This retrospective study involved 30 consecutive patients with univentricular hearts who underwent bilateral pulmonary artery banding (bil-PAB) and BDG anastomosis between September 2011 and September 2018 at our institution. These patients were classified into the nitrogen (N = 12) and no nitrogen (N = 18) groups according to their use of hypoxic gas therapy. Clinical echocardiographic, operative, and catheter examination data were reviewed. Nakata index was 131 (110-167) in the nitrogen group and 138 (88.4-161.7) in the no nitrogen group (P = 0.8). Pulmonary vascular resistance (PVR) was 1.50 (1.30-2.4) in the nitrogen group and 138 (88.4-161.7) in the no nitrogen group (P = 0.3). There was no statistical difference between the pulmonary development in both groups. In the subgroup of hypoplastic left heart syndrome patients, there was a statistical difference. RVEDV% of normal was 232.5 (215-239) in the nitrogen group and 201 (161-209) in the no nitrogen group (P = 0.03). Pulmonary development was not significantly different when hypoxic gas therapy was performed using nitrogen vs. not using nitrogen. However, RVEDV% of normal might be larger with hypoxic gas therapy using nitrogen. Although there is no obvious systemic atrioventricular valve regurgitation exacerbation with hypoxic gas therapy, it might be useful to prevent a decrease in PVR and may also affect bil-PAB and subclinical increase in systemic atrioventricular valve regurgitation.

Mid-term outcomes of cor triatriatum repair: comparison of biventricular physiology and univentricular physiology.

BACKGROUND: Cor triatriatum is the rarest of all congenital cardiac diseases, accounting for 0.1-0.4% of congenital heart diseases. Atrial septal defect is the most common associated defect; however, cor triatriatum is sometimes associated with univentricular heart. METHODS: This single-centre retrospective study involved all patients who underwent the repair of cor triatriatum at Kobe Children's Hospital between 2000 and 2020. Twenty-four patients were required surgery. We conducted a survey of survival rate, early and late pulmonary vein stenosis in each group. RESULTS: The survival rate of 5 years after cor triatriatum resection was 100% in the biventricular group and 82.1% in the univentricular group, respectively. The free rate for pulmonary stenosis of 5 years after surgery was 100% in the biventricular group and 90.0% in the univentricular group, respectively. There was no statistical difference in survival rate and 5 years free rate for pulmonary stenosis after surgery. CONCLUSIONS: The results showed that surgical correction offers good early and mid-term outcomes for both cor triatriatum with biventricular and univentricular physiologies.

Double drainage of total anomalous pulmonary venous connection revealed after surgical repair of a supracardiac total anomalous pulmonary venous connection.

Mixed type TAPVC with double drainage, where the second vertical vein enlarged rather than atrophied after surgery. If we did not recognize left-to-right shunting before surgery, it can be treated with catheterization as demonstrated by our case.

Successful balloon valvuloplasty using direct puncture of the heart for pentalogy of Cantrell with complete ectopia cordis, low birth weight, single ventricle and severe pulmonary stenosis.

We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

Computed tomography-based surgical strategy for total anomalous pulmonary venous connection.

OBJECTIVES: We used computed tomographic angiography (CTA)-based surgical planning to clarify the anatomical indications of sutureless repair technique for total anomalous pulmonary venous connection. The mid-term impact of the current surgical strategies was evaluated. METHODS: One hundred twelve patients underwent repair for total anomalous pulmonary venous connection. The study period was divided into era 1 (1996-2010, n = 56) and era 2 (2011-2018, n = 56). Patients with single ventricular heart (SVH) were included. In era 2, the indications for primary sutureless repair and branch pulmonary vein incision were based on CTA findings. RESULTS: For patients with biventricular heart, the 5-year survival was 69% and 97% in eras 1 and 2, respectively (P = 0.0024). For patients with SVH, the 5-year survival was 21% and 70% in eras 1 and 2, respectively (P = 0.0007). During the follow-up period, the evidence of post-repair pulmonary vein stenosis (PVS) was observed in 12 patients with biventricular heart [era 1, 8 patients (23%); era 2, 4 patients (13%)], and 14 patients with SVH [era 1, 6 patients (60%); era 2, 8 patients (36%)]. Using multivariable analysis, preoperative CTA was associated with improved survival in both biventricular heart and SVH and associated with post-repair PVS-free survival in SVH. Since 2011, 12 patients with post-repair PVS underwent multiple reintervention with 1 recorded death (5-year survival: 88%). CONCLUSIONS: CTA-based surgical strategy for total anomalous pulmonary venous connection provided significant survival benefit. Although post-repair PVS could occur in era 2, aggressive reintervention appeared to be associated with improved survival and vein patency.

Successful living donor liver transplantation after stent implantation in a patient with Alagille syndrome and severe bilateral pulmonary artery stenosis.

Severe pulmonary hypertension is a contraindication for liver transplantation owing to high mortality. However, decision-making regarding the treatment approach for patients with bilateral peripheral pulmonary artery stenosis, typically complicated by elevated main pulmonary artery and right ventricle pressures, can be challenging. Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.

Co-occurrence of hypertrophic cardiomyopathy and juvenile myelomonocytic leukemia in a neonate with Noonan syndrome, leading to premature death.

We report a case of a neonate with Noonan syndrome presenting with concurrent hypertrophic cardiomyopathy and juvenile myelomonocytic leukemia, which resulted in premature death. Cases with Noonan syndrome diagnosed during the neonatal period might not necessarily show mild clinical course, and premature death is a possible outcome to be considered.

Clinical assessment of diastolic retrograde flow in the descending aorta for high-flow systemic-to-pulmonary artery shunting.

OBJECTIVE: To investigate whether echocardiographic characteristics in the descending aorta of patients with cyanotic congenital heart disease who have received a systemic-to-pulmonary artery (SP) shunt can indicate shunt flow volume and predict postoperative adverse events related to high-flow shunting. METHODS: Among the 73 consecutive patients who received an SP shunt between 2010 and 2014, data for 53 patients who underwent postoperative Doppler echocardiographic assessment of diastolic retrograde flow in the descending aorta (dAo-RF) were reviewed retrospectively. RESULTS: The mean dAo-RF ratio was 0.50 ± 0.15 at intensive care unit admission and reached its peak level (0.56 ± 0.12) at 24 hours after surgery. All of the patients with a maximal dAo-RF ratio of ≥0.80 had experienced acute heart failure or cardiogenic shock due to postoperative high-flow shunting and required emergent surgical interventions to reduce pulmonary blood flow. Pulse oximetry-measured oxygen saturation and serum lactate level were significantly correlated with dAo-RF ratio, but they had some clinical dispersion to match the postoperative adverse events. CONCLUSIONS: The dAo-RF ratio is a simple, repeatable, and noninvasive index for postoperative assessment of SP shunt flow volume. A high dAo-RF ratio is a significant predictor of postoperative adverse events of high-flow shunting.

Pacing therapy in children.

BACKGROUND: The importance of ventricular pacing site in pediatric pacemaker therapy has gradually become recognized. We reviewed our experience with a left ventricular (LV)-prioritized pacing strategy. METHODS AND RESULTS: Between 2000 and 2012, 60 patients underwent 76 permanent pacemaker implantations. Eight of the 29 reoperations involved ventricular lead repositioning for pacing-induced ventricular dysfunction. Freedom from ventricular lead failure was 96.3%, 86.8%, and 81.0% at 1, 3, and 5 years, respectively. The independent predictors of ventricular lead failure were age (P=0.026) and peak minimal energy threshold within 6 months (P=0.035). At the measured points, redo bipolar, steroid-eluting leads had significantly better pacing properties than did redo non-steroid-eluting, screw-in leads (P=0.0009-0.03). Ventricular lead repositioning was effective in the 5 patients with systemic LV pacing, whereas its efficacy was inconsistent in patients with single-ventricle or systemic right ventricular (RV) pacing. At a median follow-up of 59 months, the 28 patients with LV pacing had preserved ventricular function (LV fraction shortening, 0.34±0.09). CONCLUSIONS: The outcome of this LV-prioritized pacing strategy in pediatric patients was excellent, demonstrating preserved ventricular function. Bipolar, steroid-eluting, epicardial pacing leads achieved good pacing properties, even in reoperation patients. In children with systemic LV and RV pacing-induced ventricular dysfunction, a conversion to LV apex pacing was an attractive alternative to cardiac resynchronization therapy.